We report a case of 17-month-old girl who was diagnosed with CBFA2T3/GLIS2 rearranged AMKL.Three months after a TCR αβ-CD19 depleted haploidentical HSCT she presented with decreasing donor chimaerism, positive flow MRD(0.2%) and more than two log rise in CBFA2T3/GLIS2 transcripts. After one cycle of azacitidine and venetoclax she achieved molecular remission and donor chimaerism reverted to 100%. This is the first case report of a CBFA2T3/GLIS2 rearranged AMKL responding to this combination. This strategy, if proven to be effective in a larger cohort, could be used to treat relapse after HSCT in this high risk group of patients.